Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers.

The objective of this work was to develop and validate a set of clinical criteria for the classification of patients affected by periodic fevers. Patients with inherited periodic fevers (familial Mediterranean fever (FMF); mevalonate kinase deficiency (MKD); tumour necrosis factor receptor-associated periodic fever syndrome (TRAPS); cryopyrin-associated periodic syndromes (CAPS)) enrolled in the Eurofever Registry up until March 2013 were evaluated. Patients with periodic fever, aphthosis, pharyngitis and adenitis (PFAPA) syndrome were used as negative controls. For each genetic disease, patients were considered to be 'gold standard' on the basis of the presence of a confirmatory genetic analysis. Clinical criteria were formulated on the basis of univariate and multivariate analysis in an initial group of patients (training set) and validated in an independent set of patients (validation set). A total of 1215 consecutive patients with periodic fevers were identified, and 518 gold standard patients (291 FMF, 74 MKD, 86 TRAPS, 67 CAPS) and 199 patients with PFAPA as disease controls were evaluated. The univariate and multivariate analyses identified a number of clinical variables that correlated independently with each disease, and four provisional classification scores were created. Cut-off values of the classification scores were chosen using receiver operating characteristic curve analysis as those giving the highest sensitivity and specificity. The classification scores were then tested in an independent set of patients (validation set) with an area under the curve of 0.98 for FMF, 0.95 for TRAPS, 0.96 for MKD, and 0.99 for CAPS. In conclusion, evidence-based provisional clinical criteria with high sensitivity and specificity for the clinical classification of patients with inherited periodic fevers have been developed.

Are Evaluation of ICTs Investment Using Decision Support Systems:Case Applications from Distributor's and End User's Perspective Group Decision

The evaluation of investments in advanced technology is one of the most important decision making tasks. The importance is even more pronounced considering the huge budget concerning the strategic, economic and analytic justification in order to shorten design and development time. Choosing the most appropriate technology requires an accurate and reliable system that can lead the decision makers to obtain such a complicated task. Currently, several Information and Communication Technologies (ICTs) manufacturers that design global products are seeking local firms to act as their sales and services representatives (called distributors) to the end user. At the same time, the end user or customer is also searching for the best possible deal for their investment in ICT's projects. Therefore, the objective of this research is to present a holistic decision support system to assist the decision maker in Small and Medium Enterprises (SMEs) - working either as individual decision makers or in a group - in the evaluation of the investment to become an ICT's distributor or an ICT's end user. The model is composed of the Delphi/MAH (Maximising Agreement Heuristic) Analysis, a well-known quantitative method in Group Support System (GSS), which is applied to gather the average ranking data from amongst Decision Makers (DMs). After that the Analytic Network Process (ANP) analysis is brought in to analyse holistically: it performs quantitative and qualitative analysis simultaneously. The illustrative data are obtained from industrial entrepreneurs by using the Group Support System (GSS) laboratory facilities at Lappeenranta University of Technology, Finland and in Thailand. The result of the research, which is currently implemented in Thailand, can provide benefits to the industry in the evaluation of becoming an ICT's distributor or an ICT's end user, particularly in the assessment of the Enterprise Resource Planning (ERP) programme. After the model is put to test with an in-depth collaboration with industrial entrepreneurs in Finland and Thailand, the sensitivity analysis is also performed to validate the robustness of the model. The contribution of this research is in developing a new approach and the Delphi/MAH software to obtain an analysis of the value of becoming an ERP distributor or end user that is flexible and applicable to entrepreneurs, who are looking for the most appropriate investment to become an ERP distributor or end user. The main advantage of this research over others is that the model can deliver the value of becoming an ERP distributor or end user in a single number which makes it easier for DMs to choose the most appropriate ERP vendor. The associated advantage is that the model can include qualitative data as well as quantitative data, as the results from using quantitative data alone can be misleading and inadequate. There is a need to utilise quantitative and qualitative analysis together, as can be seen from the case studies.

Customization, transparency and proximity: a user- centered content strategy applied to the design of a virtual library website

We present a case study of the redesign of the organizational presentation and content of the Virtual Library website at the Universitat Oberta de Catalunya (Open University of Catalonia, UOC), based on a user-centered design strategy. The aim of the redesign was to provide users with more intuitive, usable and understandable content (textual content, resources and services) by implementing criteria of customization, transparency and proximity. The study also presents a selection of best practices for applying these criteria to the design of other library websites.

Assessment of critical competences required in managing end-user driven businesses in a global forest products compa

Työn pääasiallisena tavoitteena on selvittää ydinosaamista, jota tarvitaan kansainvälisen metsäteollisuusyrityksen loppukäyttäjävetoisessa liiketoiminnassa. Lisäksi tavoitteena on laatia kompetenssimalli, jota voidaan käyttää mm. rekrytoinnissa, koulutuksen ja työnkierron suunnittelussa. Tarkoituksena on tarkastella sekä tällä hetkellä että tulevaisuudessa tarvittavia ydinosaamisia. Työ keskittyy myyntihenkilöiden kompetenssien kuvaamiseen. Kirjallisuuden ja asiantuntijoiden haastattelujen avulla on selvitetty. kompetensseihin liittyviä asioita ja käsitteitä, kuten organisaation oppiminen, innovaatiot sekä asiakaslähtöisyys. Tämän jälkeen on kartoitettu case-yrityksen kartonkiyksikön myyntihenkilökunnan kompetensseja. Kompetenssikartoitus on tehty teemahaastattelujen avulla sekä lisäksi on käytetty työn aikana laadittua kompetenssimatriisia. Saatuja tuloksia on verrattu yhden asiakkaan näkemyksiin. Tulosten mukaan kolme tärkeintä tämän hetken kompetenssia ovat: holistinen näkemys asioista, suhteiden rakentaminen ja kielitaito. Tulevaisuuden tärkeimpiä kompetensseja puolestaan ovat: asiakaslähtöisyys, sähköinen kaupankäynti ja suhteiden rakentaminen. Lopuksi on käsitelty yleisiä metsäteollisuuden tulevaisuuden haasteita sekä annettu joitain kehitysideoita kompetenssien kehittämistä varten.

Portable Application User Interface Development in Symbian OS

Symbian OS on käyttöjärjestelmä edistyneille matkapuhelimille. Symbiania käyttävistä laitteista on monia muunnoksia. Joitakin käytetään näppäimistön avulla ja toisia kynällä. Näytön mittasuhteet ja muut ominaisuudet vaihtelevat huomattavasti älypuhelimista kommunikaattoreihin. Tämän seurauksena Symbianin eri laiteperheiden viitemallien käyttöliittymäosat ovat sovelluskehittäjän kannalta melko erilaisia. Esimerkiksi kaikki käyttöliittymäkomponentit eivät ole saatavilla kaikissa laiteperheissä. Perinteisesti sovellusten käyttöliittymät on kirjoitettu erikseen kullekin laiteperheelle, mikä pidentää kehitysaikaa. Tämä työ esittelee Symbianin käyttöliittymäarkkitehtuurin, siirrettävyyden käsitteen ja tekniikoita sovellusten suunnitteluun ja toteutukseen, joilla saavutetaan parempi siirrettävyys Symbian-ympäristössä. Työssä suunnitellaan ja toteutetaan AppTest-nimisen testaustyökalun käyttöliittymä siten, että sovellus on helposti siirrettävissä eri laiteperheisiin.

The Classification of Vitreous Seeds in Retinoblastoma and Response to Intravitreal Melphalan.

PURPOSE: To evaluate the clinical characteristics of the 3 classifications of vitreous seeds in retinoblastoma-dust (class 1), spheres (class 2), and clouds (class 3)-and their responses to intravitreal melphalan. DESIGN: Retrospective, bi-institutional cohort study. PARTICIPANTS: A total of 87 patient eyes received 475 intravitreal injections of melphalan (median dose, 30 μg) given weekly, a median of 5 times (range, 1-12 times). METHODS: At presentation, the vitreous seeds were classified into 3 groups: dust, spheres, and clouds. Indirect ophthalmoscopy, fundus photography, ultrasonography, and ultrasonic biomicroscopy were used to evaluate clinical response to weekly intravitreal melphalan injections and time to regression of vitreous seeds. Kaplan-Meier estimates of time to regression and ocular survival, patient survival, and event-free survival (EFS) were calculated and then compared using the Mantel-Cox test of curve. MAIN OUTCOME MEASURES: Time to regression of vitreous seeds, patient survival, ocular survival, and EFS. RESULTS: The difference in time to regression was significantly different for the 3 seed classes (P < 0.0001): the median time to regression was 0.6, 1.7, and 7.7 months for dust, spheres, and clouds, respectively. Eyes with dust received significantly fewer injections and a lower median and cumulative dose of melphalan, whereas eyes with clouds received significantly more injections and a higher median and cumulative dose of melphalan. Overall, the 2-year Kaplan-Meier estimates for ocular survival, patient survival, and EFS (related to target seeds) were 90.4% (95% confidence interval [CI], 79.7-95.6), 100%, and 98.5% (95% CI, 90-99.7), respectively. CONCLUSIONS: The regression and response of vitreous seeds to intravitreal melphalan are different for each seed classification. The vitreous seed classification can be predictive of time to regression, number, median dose, and cumulative dose of intravitreal melphalan injections required.

Web user interface architecture for advanced planning and scheduling systems Case: Nokia Demand / Supply Planning Applications

In modem hitec industry Advanced Planning and Scheduling (APS) systems provide the basis for e-business solutions towards the suppliers and the customers. One objective of this thesis was to clarify the modem supply chain management with the APS systems and especially concentrate on the area of Collaborative Planning. In order Advanced Planning and Scheduling systems to be complete and usable, user interfaces are needed. Current Visual Basic user interfaces have faced many complaints and arguments from the users as well as from the development team. This thesis is trying to analyze the reasons and causes for the encountered problems and also provide ways to overcome them. The decision has been made to build the new user interfaces to be Web-enabled. Therefore another objective of this thesis was to research and find suitable technologies for building the Web-based user interfaces for Advanced Planning and Scheduling Systems in Nokia Demand/Supply Planning business area. Comparison between the most suitable technologies is made. Usability issues of Web-enabled user interfaces are also covered. The empirical part of the thesis includes design and implementation of a Web-based user interface with the chosen technology for a particular APS module that enables Collaborative Planning with suppliers.

Genotypic classification of patients with Wolfram syndrome: insights into the natural history of the disease and correlation with phenotype

Purpose: Wolfram syndrome is a degenerative, recessive rare disease with an onset in childhood. It is caused by mutations in WFS1 or CISD2 genes. More than 200 different variations in WFS1 have been described in patients with Wolfram syndrome, which complicates the establishment of clear genotype-phenotype correlation. The purpose of this study was to elucidate the role of WFS1 mutations and update the natural history of the disease. Methods: This study analyzed clinical and genetic data of 412 patients with Wolfram syndrome published in the last 15 years. Results: (i) 15% of published patients do not fulfill the current ­inclusion criterion; (ii) genotypic prevalence differences may exist among countries; (iii) diabetes mellitus and optic atrophy might not be the first two clinical features in some patients; (iv) mutations are nonuniformly distributed in WFS1; (v) age at onset of diabetes mellitus, hearing defects, and diabetes insipidus may depend on the patient"s genotypic class; and (vi) disease progression rate might depend on genotypic class. Conclusion: New genotype-phenotype correlations were established, disease progression rate for the general population and for the genotypic classes has been calculated, and new diagnostic criteria have been proposed. The conclusions raised could be important for patient management and counseling as well as for the development of treatments for Wolfram syndrome.

Classification-Based compression of Multispectral Images

The purpose of this thesis is to present a new approach to the lossy compression of multispectral images. Proposed algorithm is based on combination of quantization and clustering. Clustering was investigated for compression of the spatial dimension and the vector quantization was applied for spectral dimension compression. Presenting algo¬rithms proposes to compress multispectral images in two stages. During the first stage we define the classes' etalons, another words to each uniform areas are located inside the image the number of class is given. And if there are the pixels are not yet assigned to some of the clusters then it doing during the second; pass and assign to the closest eta¬lons. Finally a compressed image is represented with a flat index image pointing to a codebook with etalons. The decompression stage is instant too. The proposed method described in this paper has been tested on different satellite multispectral images from different resources. The numerical results and illustrative examples of the method are represented too.

Evaluation of an eLearning pilot course based on user experiences: corporate case study

Diplomityön tavoitteena oli arvioida sähköisen oppimisen soveltuvuutta kohdeyrityksessä ja selvittää, voidaanko luokkahuonekoulutusta korvata sähköisen oppimisen kursseilla. Tietojärjestelmän raportoinnista tehtiin sähköisen oppimisen kurssi, joka oli koekäytössä. Koekäytön jälkeen tehtiin käyttäjäkysely, kerättiin käyttötietoja kurssista ja tehtiin haastatteluja. Koekäyttäjien kokemuksista tehdyn arvioinnin perusteella sähköinen oppiminen soveltuu käytettäväksi selkeiden asioiden koulutukseen kohdeyrityksessä, mutta se ei voi kokonaan korvata luokkahuonekoulutusta. Luokkahuonekoulutuksessa tulisi keskittyä monimutkaisempiin asioihin ja ongelmanratkaisuun. Positiivisten tulosten perusteella sähköisen oppimisen kehittämistä päätettiin jatkaa yrityksessä. Sähköisen oppimisen kurssin avulla saadaan kustannussäästöjä kohdeyrityksessä, kun käyttäjämäärä on suurempi kuin 66. Jos koko koekäytössä olleen kurssin kohdeyleisö suorittaa kurssin sähköisesti, ovat kustannukset vain noin 15% vastaavista kustannuksista luokkahuoneessa järjestettynä. Lisäksi sähköisen oppimisen tehokkuutta tutkittiin ja koekäytössä olleen kurssin arvioitiin olevan positiivinen työssä kehitetyn Consensus-mallin mukaan.